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Secondary Leukemia in Primary Mediastinal Large B Cell Lymphoma (PMBL) Presenting as Myeloid Sarcoma in the Paraspinal Area FREE TO VIEW

Arun Kumar Arumugam Raajasekar, MBBS; Anuradha Belur, MBBS; Abhinav Chandra, MBBS
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Maimonides Medical Center, Brooklyn, NY

Chest. 2014;146(4_MeetingAbstracts):666A. doi:10.1378/chest.1991498
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SESSION TITLE: Cancer Student/Resident Case Report Posters III

SESSION TYPE: Medical Student/Resident Case Report

PRESENTED ON: Tuesday, October 28, 2014 at 01:30 PM - 02:30 PM

INTRODUCTION: Myeloid sarcoma is a localized extramedullary tumor composed of immature myeloid cells. It can occur in a patient with no prior history of acute myeloid leukemia[1]. It is usually seen in association with acute leukemia, myeloproliferative neoplasm, and myelodysplastic syndromes. They have a predilection for the skin, lymph nodes, small intestine, spine, and the mean time between the diagnosis and development of AML 10.5 months [2]. In addition MS may be the initial manifestation of relapse of AML following chemotherapy or hematopoietic stem-cell transplantation [3].

CASE PRESENTATION: A 28 year old male ppresented 4 years with chest pain to the ED. He had completed 6 cycles of R-CHOP for B-Cell lymphoma of the anterior mediastinum with subsequent follow up PET scans negative for reccurence. Initial investigations including Echocardiogram, CT chest with contrast, MRI spine failed to reveal any evidence of disease. A repeat PET/CT showed increased uptake in anterior mediastinal soft tissue and pasraspinal area compared with the prior imaging. He underwent paraspinal mass biopsy which showed myeloid sarcoma. Bone marrow biopsy and aspirate was consistent with nonpromyelocytic acute myeloid leukemia. FISH analysis was positive for a RUNX1T1/RUNX1 (ETO/AML1) rearrangement and negative for a rearrangement or loss/gain of MLL. Trisomy 11/11q, was observed in 3.8% of cells. No PML/RARA translocation were found. Karyotype showed 45,X,-Y,t(8;21)(q22;q22)(19)/46,XY(1). Patient was referred for an allogenic transplant.

DISCUSSION: Therapy-related myelodysplasia (tMDS) and secondary acute myelogenous leukemia (sAML) are late complications of high-dose therapy for lymphoid malignancies (incidence 5% to 15%). tMDS and sAML are clinically and cytogenetically distinct from de novo cases and the observed cytogenetic changes are often characteristic of chemotherapy-induced chromosomal damage . Alkylating agents are associated with complete or partial deletions of chromosome 5 and/or 7. Topoisomerase II inhibitors are typically associated with balanced translocations involving chromosome bands 11q23 and 21q22. Trisomy 11 has also been reported in myeloid sarcoma.The presence of translocation t(8;21) is associated with a relatively good prognosis when treated with standard induction and intensive consolidation chemotherapy.

CONCLUSIONS: We describe a rarely reported t(8,21) translocation in a patient with tMDS secondary to Topoisomerase II inbitor.

Reference #1: Brunning RD et al Pathology and Genetics of Tumours of Haematopoietic and Lymphoid Tissues. Vol. 3. Lyon, France: IARC Press; 2001. pp. 104-105.

Reference #2: Breccia M et al. Granulocytic sarcoma with breast and skin presentation: a report of a case successfully treated by local radiation and systemic chemotherapy. Acta Haematologica. 2000;104(1):34-37

Reference #3: Armitage JO et al. Treatment-related myelodysplasia and acute leukemia in non-Hodgkin’s lymphoma patients. Journal of Clinical Oncology. 2003;21(5):897-906.

DISCLOSURE: The following authors have nothing to disclose: Arun Kumar Arumugam Raajasekar, Anuradha Belur, Abhinav Chandra

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